Isolated café-au-lait macules: Think of neurofibromatosis type V

Sir, Segmental neurofibromatosis (SNF) is a rare form of neurofibromatosis (NF) with a reported prevalence of 0.0014%–0.002%. It is included in Riccardi’s classification as type V NF [1]. It is characterized by café-au-lait macules and/or neurofibromas distributed in only one dermatome, less commonly in two or more dermatomes [2]. Roth et al. reclassified SNF into four subtypes: true SNF, localized SNF with deep involvement, hereditary SNF, and bilateral SNF. A limited number of cases of SNF have been reported with systemic involvement, such as visceral neurofibromas, skeletal abnormalities, and renal agenesis [1]. Laser therapy may be performed if an aesthetic demand arises. Herein, we report a case of SNF in a young boy with no systemic disease. An eleven-year-old male was sent by his pediatrician for skin spots that appeared at the age of four. The young patient came from a non-consanguineous marriage and had no family history of skin disease. A general physical examination revealed normal parameters, such as weight, size, intelligence, speech, auditory function, and visual acuity. A dermatological examination found unilateral café-au-lait macules with a ranging size of 1 to 5 mm in the right half of the trunk with no crossing of the midline (Figs. 1a and 1b). We thoroughly examined the boy and found no other features of neurofibromatosis, including neurofibromas, neurological deficits, or bone abnormalities. We assured the patient and his parents that this was a benign disease, so the risk of developing any disease-related complications was low. Annual monitoring was started. No therapy was proposed because the patient expressed no aesthetic demands.

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A clinico-aetiological study of diaper area dermatoses in children

International Journal of Research in Dermatology ◽

10.18203/issn.2455-4529.intjresdermatol20181814 ◽

2018 ◽

Vol 4 (2) ◽

pp. 157

Author(s):

Bindushree R. ◽

Raghavendra B. N. ◽

Rajashekhar T. S. ◽

K. N. V. Prasad

Keyword(s):

Systemic Disease ◽

Paediatric Population ◽

Infection Group ◽

General Physical Examination ◽

Dermatological Examination ◽

Gram Staining ◽

Broad Term ◽

Culture Sensitivity ◽

Skin Conditions ◽

General Physical

Background: Diaper area dermatoses is a broad term used to describe various skin conditions that can occur in diaper area. Dermatoses in diaper area can occur as primary disease, as part of a generalised inflammatory skin disease, or as part of a systemic disease.Methods: One hundred and thirty children below five years with eruptions involving the diaper area were enrolled in the study. A detailed history, general physical examination and dermatological examination was done and recorded in a proforma. Tests such as Tzanck smear, skin biopsy, KOH preparation, culture sensitivity, Gram staining and appropriate hematological investigations were done whenever necessary depending on the presenting condition.Results: A total of twenty three (23) various dermatoses were encountered. Irritant contact dermatitis (22.3%) was the most common dermatoses, followed by scabies (14.6%), impetigo (13.8%) and papular urticaria (11.5%). Aetiological analysis revealed that majority (28.5%) of dermatoses belonged to infection group followed by inflammatory (26.15%) and arthropod bite (26.15%) groups. Amongst the infective dermatoses, bacterial infection (14.61%) was most common entity followed by viral (10.76%) and fungal infection (3.1%).Conclusions: Our study emphasizes various dermatoses in diaper area in paediatric population and proves that aetiology is multifactorial and is not because of diaper alone.

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Persistent Lower Lid Swelling in an Infant – Impacted Foreign Body!

Pakistan Journal of Ophthalmology ◽

10.36351/pjo.v36i3.1052 ◽

2020 ◽

Vol 36 (3) ◽

Author(s):

Javeria Nasir ◽

Anum Javed ◽

Owais Arshad ◽

Mohammad Hanif` Chatni

Keyword(s):

Foreign Body ◽

Physical Examination ◽

General Practitioners ◽

Foreign Bodies ◽

General Physical Examination ◽

Care Givers ◽

Impacted Foreign Body ◽

General Physical ◽

The Right ◽

In Infants And Children

Ophthalmologists, including general practitioners definitely encounter ocular foreign bodies in their clinics. Theconjunctival fornices are potential sites of impaction. We report a case of a 9-month infant boy who was referred to us for a persistent lower lid swelling for one month. He had already been to an eye specialist before presenting to us. Upon examination, a round, pink coloured, toy cart-wheel came out of his lower eye lid of the right eye. Surprisingly, there was no associated conjunctival or adnexal damage. The authors wish to emphasize the importance of taking a thorough history and adequate general physical examination. A missing part of a toy, elucidated on history, should always raise the suspicion among parents and/or care givers for a probable foreign body in infants and children.

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A CASE OF BILATERAL RENAL B-CELL LYMPHOMA WITH UVEITIS AND SECONDARY POLYCYTHEMIA IN A JACK RUSSELL TERRIER

Taiwan Veterinary Journal ◽

10.1142/s1682648520720038 ◽

2021 ◽

pp. 1-8

Author(s):

Shu-Fang Yang ◽

Heng-Leng Yang ◽

Hui-Wen Chang ◽

Yu-Han Hsieh ◽

Peng-Chen Wang ◽

...

Keyword(s):

B Cell ◽

Vision Loss ◽

Clinical Signs ◽

B Cell Lymphoma ◽

Cell Marker ◽

Abdominal Ultrasonography ◽

General Physical Examination ◽

General Physical ◽

The Right ◽

End Stage

An 8-year-old, castrated male, Jack Russell Terrier was presented with clinical signs of uveitis and glaucoma in the right eye (oculus dexter (OD)) for 2–3 months. Ophthalmic examination revealed OD episcleral congestion, corneal vascularization, aqueous flare, iris bombe, and vision loss. OD glaucoma and uveitis were under control by medical treatment. After 8 months, the dog had acute uveitis in the left eye (oculus sinister (OS)). General physical examination, blood examinations, radiography, and urinalysis were unremarkable. No improvement was observed after for 1 month of uveitis treatment, and more examinations were strongly suggested. Abdominal ultrasonography revealed several hypoechoic masses or cysts on both kidneys. Computed tomography (CT) showed diffuse nonenhancing hypodense patches in both kidneys. Renal tumor was highly suspected, but biopsy was declined by the owner. Ten days after CT examination, the dog suddenly developed anorexia, weakness, uremia, hyperkalemia, and polycythemia. The dog was euthanatized because of the worsening clinical condition, poor prognosis, and financial constraints, and both kidneys were biopsied for histopathology. Immunohistochemically, the neoplastic cells were positive for B-cell marker (CD20) and negative for T-cell marker (CD3). Bilateral renal B-cell origin lymphoma was diagnosed by histologic and immunophenotyping examinations. In conclusion, bilateral renal lymphoma may be diagnosed with no systemic signs except bilateral uveitis. Renal function impairment was acutely developed with severe clinical signs at the end stage of the disease. Early, aggressive, complete examinations, and treatments are strongly recommended.

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Multifocal granulocytic sarcoma of the spine mimicking neurofibromatosis Type 2: case report

Journal of Neurosurgery Spine ◽

10.3171/2016.2.spine151044 ◽

2016 ◽

Vol 25 (4) ◽

pp. 523-527

Author(s):

Gregory P. Lekovic ◽

Marc S. Schwartz ◽

John L. Go

Keyword(s):

Granulocytic Sarcoma ◽

Neurofibromatosis Type ◽

Rapid Onset ◽

Neurofibromatosis Type 2 ◽

Neurological Deficits ◽

Lumbosacral Spine ◽

Imaging Features ◽

History Of ◽

Patient’S History

In this report the authors report on a patient with a very indolent course of granulocytic sarcoma, characterized by steroid-induced remission of spinal and cranial tumors and recurrence over a period of several years. This 24-year-old man with history of leukemia presented with rapid-onset quadriparesis secondary to multiple extraaxial masses of the cervicothoracic spine, from C-5 to T-3, and lumbosacral spine, from L-5 to the coccyx. Although the imaging features were highly suggestive of neurofibromatosis Type 2, the patient's history and clinical course were consistent with granulocytic sarcoma; repeat imaging and, later, needle biopsy definitively established the diagnosis of granulocytic sarcoma. Laminectomy and surgical decompression of the spine were not required and, arguably, could have posed unnecessary risk to the patient. This case illustrates that the successful management of a patient presenting with profound neurological deficits due to intradural spinal cord tumors may sometimes be nonsurgical.

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Parasagittal meningioma: A not so benign entity

Medicine Science and the Law ◽

10.1177/0025802417732267 ◽

2017 ◽

Vol 57 (4) ◽

pp. 175-178 ◽

Cited By ~ 2

Author(s):

Roger W Byard

Keyword(s):

Neurofibromatosis Type ◽

Lethal Effect ◽

Precentral Gyrus ◽

Unexpected Death ◽

Comfort Care ◽

Posterior Displacement ◽

History Of ◽

The Right ◽

Parasagittal Meningioma

While the majority of intracranial meningiomas have an indolent clinical course, sudden and unexpected death may rarely occur. Two cases are reported to demonstrate rapid clinical deterioration resulting in death in individuals with large, grade I, parasagittal meningiomas. Case 1 was a 46-year-old man with a history of headaches and epilepsy who suddenly collapsed and died. A large right frontal parasagittal meningioma with haemorrhage had compressed the brain and lateral ventricle, causing tonsillar herniation. In case 2, a previously well 83-year-old woman presented with a one-week history of progressive dysphagia and dysphasia. She suffered rapid deterioration and was prescribed comfort care. A right-sided parasagittal meningioma had compressed the right superior and middle frontal gyri with posterior displacement and compression of the right precentral gyrus. If a meningioma is found at autopsy, the possibility of a lethal effect should be considered, and evidence of neurofibromatosis type 2 or other associated heritable conditions checked for.

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Bilateral Subdural Haematoma and CPAP Use: A Possible Association

European Journal of Case Reports in Internal Medicine ◽

10.12890/2020_001602 ◽

2020 ◽

Author(s):

Beatrice Khater ◽

Vicky Kassouf ◽

Georges Haddad ◽

Roula Hourany

Keyword(s):

Subdural Haematoma ◽

Brain Mri ◽

Sleep Apnoea ◽

Neurological Deficits ◽

Angiotensin Ii Receptor Blocker ◽

Angiotensin Ii Receptor ◽

Acute Subdural Haematoma ◽

Obstructive Sleep ◽

History Of ◽

The Right

Obstructive sleep apnoea (OSA) is a common condition usually treated with continuous positive airway pressure (CPAP). No reports have linked it to an acute subdural haematoma. A 54-year-old white man who had hypertension well controlled with an angiotensin II receptor blocker, presented with a 2-week history of occipital headache with no other focal neurological symptoms. The headache began 12 days after he had started using CPAP for OSA. A brain MRI performed 2 weeks later showed bilateral subdural haematomas which were chronic on the left and sub-acute/acute on the right. Since the patient was clinically stable with no focal neurological deficits, he received prednisone for 3 weeks and was followed up with consecutive CT scans demonstrating gradual regression of the haematomas. This is the first report showing that subdural haematomas could be linked to CPAP use.

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Purtscher’s retinopathy: a case report

Journal of Chitwan Medical College ◽

10.3126/jcmc.v10i3.32066 ◽

2020 ◽

Vol 10 (3) ◽

pp. 103-105

Author(s):

Sushma Chaudhary ◽

Sharad Gupta ◽

Reena Yadav ◽

Vinit Kumar Kabmle ◽

Satbodh Shrestha

Keyword(s):

Systemic Disease ◽

Fluorescence Angiography ◽

Purtscher’S Retinopathy ◽

Corrected Visual Acuity ◽

History Of ◽

Anterior Segments ◽

The Right ◽

Sudden Blindness ◽

Time Of Presentation ◽

Definite Treatment

Purtscher’s retinopathy is a rare haemorrhagic and vaso-occlusive vasculopathy following severe head trauma associated with sudden blindness. Here we report a case of 28 years old male presented to out-patient department with history of sudden and painless loss of vision in his right eye for two days. He gave history of head injury following bike accident. At the time of presentation his best corrected visual acuity was 1/60 in the right eye and 6/6 in the left eye. On examination, anterior segments of both eyes were normal. On fundus examination, right eye revealed retinal whitening in the peripapillary region (Purtscher’s flecken), an intraretinal hemorrhage and signs of macular edema whereas left eye was unremarkable. Findings were suggestive of right eye purtscher’s retinopathy. Optical coherence tomography and fundus fluorescence angiography was done to support the diagnosis. Purtscher’s retinopathy should not be neglected because it is associated with different systemic disease. Its pathophysiology remains unclear and there is no definite treatment but meticulous management is urgent to prevent from severe complications.

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“Unusual brain stone”: heavily calcified primary neoplasm with some features suggestive of angiocentric glioma

Journal of Neurosurgery ◽

10.3171/2014.11.jns131158 ◽

2015 ◽

Vol 123 (5) ◽

pp. 1256-1260 ◽

Cited By ~ 1

Author(s):

Jahangir Sajjad ◽

Chandrasekaran Kaliaperumal ◽

Niamh Bermingham ◽

Charles Marks ◽

Catherine Keohane

Keyword(s):

Ct Scan ◽

Neurological Deficits ◽

Parietal Region ◽

History Of ◽

Angiocentric Glioma ◽

Primary Neoplasm ◽

History Of Epilepsy ◽

The Right ◽

Visual Blurring ◽

Excised Tissue

This 40-year-old man presented with a 5-month history of progressive right-sided headache associated with visual blurring. He also had a history of epilepsy but had been seizure free with medication for the past 10 years. An initial CT scan of his brain performed 16 years previously had revealed a small area of calcification in the right parietal region. In the current presentation, he had a left-sided homonymous hemianopia but no other neurological deficits. A CT scan of his brain showed a much larger calcified, partly cystic lesion in the right parietal region. Because he was symptomatic, the lesion was excised and the cyst was drained. Histological examination of the excised tissue showed an unusual primary tumor that was difficult to classify but had some features of angiocentric glioma. The heavy calcification, mixed-density cell population, and regions with features of angiocentric glioma were most unusual. The patient remained asymptomatic 5 years after surgery, and follow-up scans did not show recurrence.

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Male with rhinosporidiosis of urethra: A case report

Bangabandhu Sheikh Mujib Medical University Journal ◽

10.3329/bsmmuj.v8i2.28936 ◽

2016 ◽

Vol 8 (2) ◽

pp. 128

Author(s):

Tanvir Ahmed ◽

Faruk Hossain ◽

Mohammad Ershad Ahsan Sohel ◽

Mohammad Al Amin ◽

A.K.M. Khurshidul Alam ◽

...

Keyword(s):

Inflammatory Cells ◽

Routine Examination ◽

General Physical Examination ◽

The Past ◽

Urethral Orifice ◽

History Of ◽

Water Pond ◽

General Physical ◽

Local Examination

A 45-year old male presented with a six months history of a polypoidal lesion at external urethral meatus. There was a history of dysuria with bloody discharge from the lesion. The past and personal history was insignificant except for occasional bathing in a water pond. General physical examination and examination of nose, oral cavity and eyes was unremarkable. On local examination, a small red, fleshy, sessile lesion was seen at the external urethral orifice measuring 0.7 x 0.6 x 0.2 cm, clinically resembling a urethral caruncle. Urine routine examination showed presence of red cells. The lesion was completely resected under spinal anaesthesia. Histopathology showed stratified squamous lined tissue which reveals many sporangia containing endospore. Subepithelial region shows granulation tissue and is infiltrated with chronic inflammatory cells suggestive ofrhinosporidiosis. No other treatment was given. Patient was well after 3 months with no evidence of recurrence during follow up.

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Clinical study of geriatric dermatoses

International Journal of Research in Dermatology ◽

10.18203/issn.2455-4529.intjresdermatol20190231 ◽

2019 ◽

Vol 5 (1) ◽

pp. 128

Author(s):

M. Vairaprabha Devi ◽

N. C. Manikandan

Keyword(s):

Outpatient Clinic ◽

Healthy Life ◽

Dermatological Examination ◽

Number Of Patients ◽

Male Preponderance ◽

History Of ◽

Study Population ◽

Benign Tumours ◽

General Physical ◽

A Prospective Study

Background: Diagnosis of geriatric dermatoses is a challenging job for the Physician due to the involvement of many intrinsic and extrinsic ageing factors. The present study was done with the aim to determine the clinical profile and pattern of dermatological manifestations in elderly people aged 60 and above and to analyze the correlation of various geriatric dermatoses with systemic diseases.Methods: This was a prospective study done on 200 elderly individuals aged 60 years and above attending the outpatient clinic of Department of Dermatology, RGGGH and the outpatient clinic of Department of Geriatrics, RGGGH during the study period from October 2015 to September 2016. Detailed history of cutaneous complaints, present and past medical complaints were taken. A complete general, physical, systemic and dermatological examination was done in all patients and the findings were noted and analysed.Results: Male preponderance was observed (M:F-1.8:1). Maximum number of patients (n=166) belonged to the age group of 60-69 years. Wrinkling of the skin is the commonest physiological geriatric dermatoses. Among the pathological changes, infections and infestations were observed in 27.5% of the study population. Fungal infection was the commonest among them (20%) followed by bacterial (18%) and viral (12%). Diabetes Mellitus was the commonest associated systemic ailment and was observed in about 31.5% of the study population.Conclusions: Geriatric dermatoses occur in the elders due to extrinsic and intrinsic ageing factors. Crinkles were the commonest physiological geriatric dermatoses and benign tumours were the commonest pathological ones. Educating the elders about proper skin care along with the early diagnosis and treatment of their cutaneous ailments would help them to lead a productive and healthy life.

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